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2016 National Child Count of Children and Youth Who Are Deaf-Blind Report

Etiologies

Over 75 specific etiologies are identified in the 2016 National Deaf-Blind Child Count. Some are exceedingly rare, with less than five cases. Others are much more prevalent. There are 13 etiologies with at least 100 cases, including Goldenhar Syndrome, added this year.

Complications from prematurity continues to be the most commonly identified etiology. The prevalence of CHARGE Syndrome continues to increase. In 2016 there were 924 children and youth identified as having CHARGE Syndrome. While still relatively rare, the prevalence of children with Stickler Syndrome and Dandy Walker Syndrome has also increased. About 18.5% of the children and youth have no determined etiology.

Overall, the total number of children and youth identified with a prenatal congenital or postnatal non-congenital etiology has declined from 2,870 to 2,516 over the past seven years, while the number with hereditary syndromes and disorders has increased from 3,584 to 4,277. Hereditary syndrome and disorders now account for 44.4% of all identified etiologies. Prenatal congenital complications account for 14.3% of identified etiologies and postnatal non-congenital complications account for 11.8% of identified etiologies. Complications from prematurity account for 10.7% of identified etiologies and 18.8% of the children and youth have no identified etiology.

Congenital Rubella Syndrome (CRS), which has long been associated with the development of the deaf-blind technical assistance system, is no longer a prevalent etiology. In 2016, there were 47 individuals whose etiology was identified as CRS, down from 89 in 2010. Over 75% of these individuals are over 10 years of age and over 50% are 15 years of age or older.

The heterogeneous nature of the population cannot be overstated. The etiologies or causes of deaf-blindness bring unique issues and challenges. Some are relatively more common, such as CHARGE Syndrome, Down Syndrome, Usher Syndrome, and cytomegalovirus. Some are very rare, with less than five individuals reported nationally, such as Batten disease, Prader-Willi, and Kearns-Sayre Syndrome.

Primary Identified Etiology

  • Hereditary Syndromes/Disorders
    • CHARGE syndrome

      924

    • Usher syndrome (I,II,III)

      296

    • Down syndrome (Trisomy 21 syndrome)

      305

    • Stickler syndrome

      133

    • Dandy Walker syndrome

      111

    • Goldenhar syndrome

      107

    • OTHER: Hereditary Syndrome/Disorders

      2401

  • Prenatal/Congenital Complications
    • Cytomegalovirus (CMV)

      292

    • Hydrocephaly

      213

    • Microcephaly

      208

    • OTHER: Pre-Natal/Congenital Complications

      666

  • Postnatal/Non-Congenital
    • Asphyxia

      199

    • Severe Head Injury

      161

    • Meningitis

      135

    • OTHER: Post-Natal/ Non-Congenital

      642

  • Complication of Prematurity

    1028

  • No Determination of Etiology

    1814